Budd-Chiari syndrome
ICD-10 I82.0 · ICD-11 DB98.5

When Early TIPS Fails in Budd-Chiari Syndrome with Long-Segment Hepatic Vein Occlusion

In Budd-Chiari syndrome characterised by long-segment thrombotic occlusion of the hepatic veins — presenting with liver failure, ascites, or varices — early portosystemic shunting is the first escalation step. When that intervention does not reach its haemodynamic and renal targets within two weeks, a further treatment line is indicated.

Clinical scenario
Long-segment thrombotic occlusion of the hepatic veins with symptomatic presentation: liver failure, ascites, or varices. This pattern is among the more severe forms of Budd-Chiari syndrome and may require escalation beyond initial shunting procedures.
Previous line — failure condition
Early transjugular intrahepatic portosystemic shunt (TIPS) did not achieve the target portosystemic pressure gradient of approximately 10.8 mmHg or the target creatinine of approximately 0.8 mg/dL within two weeks. Non-response to TIPS is the trigger for escalation to this protocol.
Next treatment approach
The protocol for this situation specifies liver transplantation as the next step; patient selection criteria, timing, and the complete clinical pathway are available in the full protocol below.
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References

DOI: 10.3390/diagnostics13081458 The long-segment thrombotic occlusion of hepatic veins, common in Western countries, is more severe and may require a portocaval shunting procedure to relieve hepatic and splanchnic congestion. In symptomatic patients (liver failure, ascites, varices), early TIPSs may be indicated without waiting on their response to medical treatment. Liver transplantation performed during the MELD era delivers results comparable to TIPS with actuarial overall survival rates of 76–85%, 71%, and 68% at one year, five years, and ten years, respectively. View source ↗